This scientific study investigated the use of serum neurofilament light chain (sNFL), particularly when adjusted for age and BMI as z-scores (zNFL), as a biomarker for chronic inflammatory demyelinating polyneuropathy (CIDP). Analyzing a large cohort of patients with immune-mediated neuropathies, the researchers found that elevated zNFL correlated with increased disease severity in patients with typical CIDP, especially in the early stages of the illness. A zNFL score above 2 in the early phase of typical CIDP was associated with more severe motor dysfunction and a lack of response to initial standard treatments, suggesting that this biomarker could help identify patients needing more aggressive, early immunotherapy and potentially distinguish between typical and atypical forms of CIDP. The study concludes by recommending sNFL measurement with z-score analysis as a valuable tool for CIDP diagnosis, assessment of severity, and treatment guidance in clinical practice.

This is a research article from the New England Journal of Medicine detailing the findings of the SELECT-GCA clinical trial, which investigated a potential new treatment for giant-cell arteritis. The trial randomly assigned patients to receive either upadacitinib at different doses or a placebo, in combination with a glucocorticoid taper. The results indicated that a 15 mg dose of upadacitinib was more effective than placebo in achieving sustained remission and reducing glucocorticoid exposure. The study also reviewed the safety profile of upadacitinib in this patient population.

This comprehensive review focuses on validated techniques used by clinicians to assess functional motor weakness, also known as functional neurological symptom disorder, which is a common presentation in neurology. The article discusses the importance of employing specific, studied examination methods to distinguish functional weakness from objective neurological weakness, which arises from structural or electrophysiological causes. Several diagnostic signs are described, including Hoover's sign, give-way weakness, the abductor sign, the abduction finger sign, the spinal injuries center test, motor inconsistency, drift without pronation, paradoxical wrist flexion, and the elbow flex-ex sign, detailing their techniques, interpretation, and limitations based on existing research. The review emphasizes that interpreting these findings should occur within a full neurological examination, potentially using multiple techniques to improve diagnostic accuracy and patient care.

This paper serves as a comprehensive review of approaches for treating neuropsychiatric symptoms in Parkinson's disease (PD). It highlights that these non-motor symptoms are highly prevalent, significantly impair quality of life for both patients and caregivers, and often appear before motor symptoms. Despite recent advancements in diagnosis and management, treatment options, including both pharmacological and non-pharmacological therapies, remain limited due to the complex underlying brain changes in PD. The review summarizes current treatments for cognitive impairment, psychosis, depression, anxiety, apathy, and impulse control disorders, while also outlining ongoing research efforts to address the existing therapeutic gaps. Ultimately, the paper emphasizes that the treating neurologist's expertise is crucial for tailoring individualized treatment plans in this challenging context.

This multicenter cohort study investigated the effectiveness of rituximab in preventing relapses in adult patients diagnosed with anti-NMDAR antibody-mediated encephalitis. Researchers evaluated the impact of a single course of rituximab on the time to first relapse and explored the duration of its effect, specifically at six and twelve months following treatment. The findings suggest that a single rituximab course is associated with a reduced risk of relapse, with the protective effect lasting for approximately six months. These results highlight the potential benefit of rituximab in managing this condition and suggest optimal redosing intervals for select patients.

This episode combines two articles and discusses the importance of laboratory expertise in accurately diagnosing autoimmune neurological syndromes, particularly autoimmune encephalitis and paraneoplastic syndromes, which are often difficult to identify due to overlapping symptoms and low prevalence. The author reviews two companion studies evaluating commercially available tissue-based assays for detecting antibodies against intracellular and surface antigens. The studies reveal significant limitations in the sensitivity and specificity of these commercial tests when used as standalone screening tools, highlighting the risk of both false positives and false negatives. Ultimately, the piece emphasizes the need for multimodal testing, skilled interpretation of results, and interdisciplinary communication between clinicians and laboratory staff to ensure reliable diagnosis and improve patient outcomes.

This paper provides a comprehensive review of repeat expansion disorders, focusing on their significance in neurology. It explains how abnormal expansions of DNA repeats cause a wide range of neurological conditions, highlighting the diagnostic challenges and opportunities for management. The authors discuss the molecular characteristics of these disorders, including repeat size, location, and inheritance patterns, and detail the clinical features like anticipation and heterogeneity that can aid in diagnosis. Finally, the piece outlines the evolution and current state of genetic testing methods, emphasizing the increasing role of whole-genome sequencing and bioinformatics tools in detecting these complex genetic variations.

This paper is a comprehensive review of IgG4-related disease (IgG4-RD) as it affects the nervous system. It discusses the clinical presentation, diagnosis, and treatment of this rare inflammatory and fibrotic condition, which can impact various parts of the nervous system, including the eyes, meninges, pituitary gland, brain parenchyma, and peripheral nerves. The article highlights the importance of early recognition and a high index of clinical suspicion due to the diverse ways IgG4-RD can manifest and mimic other conditions. While elevated serum IgG4 levels can be helpful, the paper emphasizes that histological confirmation is the gold standard for diagnosis. Treatment typically involves glucocorticoids as a first-line therapy, with other immunosuppressants like rituximab also being utilized, and a treatment algorithm for nervous system involvement is provided. The text also addresses the challenges in diagnosis due to the heterogeneity of symptoms and the ongoing need for further research and refined diagnostic criteria.